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Sunday, May 27, 2012

Meet the Band (of Brothers and Sisters)

Meet Kenan’s peers, the ones I ask special prayers for, the ones traveling the road of Krabbe Leukodystrophy along side him. With the exception of Marshall, Trevor and Anaya, they were diagnosed around the same time and are close in age, although each is experiencing the disease at a different rate. None were identified through newborn screening and none were eligible for stem cell transplant. They are an international crew, each with a story of sorrow, hope and inspiration. Social media has aloud us to come together, sharing our children’s stories, experiences and resources. Likewise, when one of us is hurting or suffering, we all are too.

Clockwise, back to front:

Lauren on Guitar - Canada
Ginevra making Devil Fingers - Italy
Trevor on Bells - California (one of fraternal twins. His brother, Tyler, is unaffected.)
Marshall on Recorder and Vocals- Oregon (diagnosed at 17-months with late infantile, a month after his little brother     Michael was born. Through Marshall’s diagnosis, Michael was identified with Krabbe and received a stem cell transplant early on. He is doing well. Another strong case for the importance of Expanded Newborn Screening.)
Gabriel on Keyboards– Mexico
Kenan on Vocals - Illinois
Jo-Jo on Guitar- Texas
Anaya- Canada: Represented by the rainbow and Gabriel's t-shirt. We lost her this past November 2011.

Our inspiration for The North Costenitos: Gabriel’s’ mother, Mayte Jimenez, an exceptional woman up against numerous odds, living in an economically strained, Mexican community with few resources, along side a medical establishment with limited-to-no knowledge at best, that has “given up” on Gabriel because his situation is deemed “hopeless”. There is no hospice nurse that comes to visit once a week, no Walgreen’s Infusion Service shipping monthly medical equipment and supplies, no medical insurance to help cover expenses, and no one to turn to when Gabriel suffers through his 200+ seizure episodes. Yet, it the midst of this, Mayte never stops having fun with her son. We all watched as she posted pictures of Gabriel in a sombrero, with sunglasses, lying in the tub, a fruity umbrella drink at his side, or when she posed him with a blow up saxophone and keyboards. Her desire to role-play was infectious. One winter afternoon, we all joined in, rummaging through toys, looking for our instrument of choice. 

Play and the exchange that comes from it is a crucial part of a child’s life and expression. Our Krabbe Kids have very little ability to support or move their bodies, they can’t talk or eat, some can’t see or hear, others struggle to breath; because of this, play is a word that is forever altered. Through Mayte and Gabriel, we had the opportunity to connect our children through a virtual, international, play date. It made for a very special afternoon for all of us. Thank you to Lauren’s dad, Stephen Loughheed, for his Photoshop skills and Tammy Wilson for working on the t-shirts (coming soon).

Below is a list of blogs, fundraising pages, and sites for the kids. A little help can go a long way:


Ginevra/Veronica Arnieri

Lauren/Amanda Zarifah

Trevor/Nicole Goreham Aldrian

Marshall/Tammy Wilson,

Anaya/Camara Cassin 

Saturday, May 19, 2012


I am happy to announce that we finally have Kenan's medications sorted out and he is no longer in a state of constant pain. All Krabbe Kids experience the same milestones of the illness, but the side effects of those symptoms vary from child-to-child. It was maddening to be patient through the process, especially when he couldn't communicate with words what he was feeling, waiting for a clue or clear sign of what was wrong. We are grateful to have that behind us.

In December, I took Kenan to see the specialist for Krabbe, Dr. Maria Escolar, at the Children's Hospital in Pittsburgh. We participated in her research study and in return, now have her staff as part of our medical team. They are an exceptional group of people and available to any concern or question we may have. I am grateful to have them, along with Horizons Hospice and Palliative Care, The Hunter's Hope Foundation, and Dr. Waggoner at Comer's Children's Hospital in our corner.

Kenan has changed a great deal since his diagnosis in October. He lost his ability to smile early on, most of his muscle tone is gone, he can no longer swallow well enough to take food by mouth - fed via g-tube, he lost his vision around his first birthday, and general activity and functions are more taxing for him now. Alternatively, his hearing is still very strong, he is able to connect with his environment, expresses his feeling through eye movements, and every once in a while, a signal will get through and he is able to make his laugh sound, indicating that cognitively he is experiencing the world like any other 15-month old. Those are the best few seconds I could ask for. He is receiving numerous therapies and we do everything we can to make sure his life is as comfortable and full as possible.

Tamsen, 3.5 years, is well. There are families who can't imagine taking care of another child in the midst of this, we can't imagine not. She is such a vital energy and her love is direct and uncomplicated. We talk honestly and openly about Kenan's symptoms as they present. Now that Kenan is comfortable and we are able to move out of crisis mode, there is a lot more cuddling and affection displayed between the two of them. We plan on attending The Hunter’s Hope Symposium as a family this summer, giving Tamsen a broader context for her brother’s struggle. 

Dann and I are also well. The emotional upheavals, of which there are many, are dealt with as they come so that we can be available to each other, our business, Tamsen and Kenan. It is simple, really: KENAN IS ALIVE! We care for him with the same devotion and diligence, sick or healthy, it doesn't matter.

Sunday, May 6, 2012

Saturday, May 5, 2012

We All Love to Eat! 
Tastes and Smells: A Major Part of the Day

We all know how uncomfortable we are at the dentist: mouth open, doctor doing work, a pool of spit gathering in the back of our throat, we struggle to swallow in an attempt to manage it. This is what it’s now like for Kenan 24/7. In November, when he was 9-months old he lost his ability to “functionally swallow”, take in nourishment by mouth without the risk of choking and/or aspirating. Since then, he’s been fed exclusively through his g-tube. Enter his speech and swallow therapist, to help us, help him, continue to explore the world and gain pleasure from oral stimulation and to keep him using those muscles for as long as possible. We offer Kenan tastes and smells throughout the day, dipping q-tips into various juices, smoothies, sauces, jams, frosting, chocolate, etc. When appropriate, he also samples what we are eating for dinner. He LOVES it and so do we. Some of his favorites: Sara Knizhik’s Bolognese sauce, barbeque sauce with hints of gouda cheese, cilantro and chicken from the barbeque chicken pizza we make (yes, very sophisticated!), hot fudge, and blueberry anything. He’s probably the only baby to sample an occasional taste of wine and beer (why the hell not?!). We move slow and observe him closely making sure there is no risk of choking. Our suction machine is always close by to assist if he becomes overwhelmed.

Tastes Picture: Hot Fudge Sauce, Blueberry Smoothie, Papaya Juice, Strawberry Jam, Odwallla Super Food , and a Cherry Sucker.

Smells Pictured: Ginger Root, Clementines, Lemons, Coffee, Chili Powder, Cocoa Powder, Cloves, and Caraway Seeds.
Written April 10, 2010

This is Kenan’s favorite position. It’s referred to as abnormal posturing, an adjective that remains difficult no matter how many time I hear it. It’s where he naturally goes and where he retreats when confronted with too much stimuli. Reasoning aside, as his mother and perhaps even as an artist, I love and admire the beauty of his body, the perfection of the arc, the strength of his muscles in sustaining the hold, his little, pointed toes. The irony is, that when relaxed, Kenan has very low muscle tone and can’t support his own body weight, another contradiction of the disease. I often refer to him as " My Little Baryshnikov".
Written March 31, 2012

Portrait of a Krabbe Kid

I am posting this picture of Kenan, taken at 9 months, to be more honest and respectful of his suffering. What’s missing is the sound: the neurological, high-pitched, forceful scream that comes from the pain of muscle spasms, hypersensitivity to touch, the irritability caused by a brain atrophying from nerve damage, the frustration of a body that won’t do what it’s meant to, and fear. Pre-medication, Kenan spent 90% of his day in this state. Post medications, 40%, 10% of which is normal baby stuff. Some days are better, others worse. 

In 2007, the State of Illinois passed a law stating they would start testing for Krabbe along with four other lysosomal disorders as part of the mandatory newborn screening panel (NBS). The program has yet to become available to the public. The hope is to have it up and running in 2012, but I have found several websites that state the same for 2009, 2010, and 2011. Part of the delay stems from an ethical debate in the medical community over testing for a disease there is no guaranteed treatment or cure for and, with the exception of *early infantile, no way of telling when a child will present with symptoms. There are 70 different mutations to the gene that can cause Krabbe and **siblings that have the exact same mutations can present differently. With all of that being true, ask any parent of an affected child, let alone two, and they will tell you there is no ethical debate in knowing and understanding your child completely in order to be fully responsible to them: transplants, future medical advancements, a cure included. So much of Kenan remained a mystery until we he had his diagnosis. 

*Kenan has two copies of the same deletion, the only one indicative of early infantile, with the recommendation of immediate transplant before the onset of symptoms. Time is critical as it takes 6 months for the body to start producing the missing enzyme post transplant. Whatever damage occurs in the meantime is permanent. Studies have shown that once symptoms present, a transplant does nothing to change the course of the illness and may, in fact, speed it up.

** Tamsen was tested. She is a carrier, like Dann and I, and will never have symptoms.

 In my last conversation with the Illinois Board of Health, it was explained to me that there are problems with the lab equipment being used for NBS and that they were looking to New York State, the only state that currently tests for Krabbe, for assistance. They could not provide me with a timeline for sorting out the issues. 

It is the span of time between the bill passing and now that is troubling, but when a group of professionals making up the Board of Health question the validity of what's occurring, you have to ask why. It is a complex issue full of contradictions and as a parent to separate from what's happening to your own child in order to understand the bigger picture, is a true act of will. I understand the danger in rushing something that is not ready, but I ask, no, I beg the Illinois Board of Health to act with a sense of urgency. 
Written March 28, 2012

Thank you, Kenan, for cracking a smile in your sleep tonight. You overwhelm me.

Went to the lake for the first time this year to listen to the waves, 3/25/12
Need I say more?

Taken on August 26, 2011 when Kenan was 6-months old. Two weeks later he would start loosing motor function in his hands. 
Written March 16, 2012

Good Morning everyone. Here is the first of weekly updates on Kenan. Although we continue to have our daily struggles with Kenan's digestion and irritability, he has gifted us these past two weeks with some deliberate attempts at vocalization. We play a game of back-and-forth sound making. He loves hearing his voice in his oversized magic echo microphone (thank you Auntie Sammy). It's been wonderful to see signs of Kenan's cognition and to have tools to pull it out. We have a very strong little boy and are very proud of him!
Written March 6, 2012

Looking Dapper In His New Hand Splints

Kenan keeps his hands fisted and clenched in a state of constant tension. Attesting to his strength, I have to try very hard to pry them open. These braces allow him to stretch and relax those muscles with the hope of making him more comfortable.
Written February 27, 2012

Kenan at 3 months. Although he would lose his ability to smile by 10 months the essence of it remains in his expressive eyes and subtle shift in vocal tone. He works through this simple gesture that you and I take granted. Although he is only a year old, I look at him and see the strength of a man.

Written February 5, 2012

At this time last year I was hours away from the birth of our son. I found this picture of him, taken at a few weeks old, when all was seemingly right in our world. Six months later he would be diagnosed with Krabbe Leukodystrophy, a fatal metabolic disorder that has treatment option if caught before symptoms appear. Had Kenan been identified through newborn screening, we'd be fighting a different fight now. Knowledge is, indeed, power. Please support Expanded Newborn Screening in your state. It saves lives and families. Happy First Birthday Kenan!